Anat Cell Biol.  2022 Jun;55(2):264-268. 10.5115/acb.21.260.

Congenital cystic adenomatoid malformation of the lung: a case report with embryological review

Affiliations
  • 1Department of Pathology, ESIC Medical College & Super Specialty Hospital, Sanathnagar, Hyderabad (Telangana), India
  • 2Department of Anatomy, All India Institute of Medical Sciences, Bibinagar, Hyderabad (Telangana), India

Abstract

Congenital cystic adenomatoid malformation (CCAM) is a rare developmental dysplastic lesion that affects the fetal bronchial tree. Etiopathogenesis is still poorly understood. Most accepted view is that of abnormal branching of bronchioles during the period of morphogenesis. We observed a rare congenital anomaly of the lungs during fetal autopsy. Routine antenatal ultrasonography revealed multiple echolucent cysts in the right lung of the fetus. Thorough external and internal examination was followed by sectioning of each organ for histopathological examination. Histopathology of the right lung showed distortion of the parenchyma with dilated bronchioles. Multiple cysts lined by columnar epithelium along with loose intervening connective tissue were observed along with many congested and dilated blood vessels. Knowledge of congenital anomalies of the respiratory system would help clinicians to plan the management at a very early stage. Accurate fetal autopsy along with clinical data is important in evaluating fetal deaths and can help in reducing unexplained stillbirths.

Keyword

Congenital; Cystic adenomatoid; Embryology; Lung malformation

Figure

  • Fig. 1 (A) Male fetus (gestational age, 24 weeks) received for autopsy. ‘I’ shaped nick given followed by opening of thoracic and abdominal cavity. (B) En bloc of lung tissue removed during autopsy, showing multiple cysts (red arrow). (C) Formalin fixed lung tissue of fetus, showing cystic wall (red arrow).

  • Fig. 2 (A) Section from lung tissue showing multiple small cystic spaces (CS) with adjacent alveoli (H&E, ×100), (B): Higher magnification image of the cyst showing cyst wall lined by columnar epithelium (red arrows) (H&E, ×400). BV, blood vessel.

  • Fig. 3 Showing classification of congenital cystic adenomatoid malformation (CCAM) according to site of origin in the tracheobronchial tree (type 0–4). Data from Stocker et al. (Hum Pathol 1977;8:155-71) [5].


Reference

References

1. Kanavi JV, Thangaraj J, Thomas A. 2017; Congenital cystic adenomatoid malformation (CCAM): antenatal and postnatal management. Int J Reprod Contracept Obstet Gynecol. 6:2447–51. DOI: 10.18203/2320-1770.ijrcog20172329.
Article
2. Stocker JT. Stocker JT, Dehner LP, editors. 2001. The respiratory tract. Pediatric Pathology. Lippincott Williams & Wilkins;Philadelphia: p. 445–517. DOI: 10.1201/9781315382531-36.
3. Langston C. 2003; New concepts in the pathology of congenital lung malformations. Semin Pediatr Surg. 12:17–37. DOI: 10.1016/S1055-8586(03)70004-3. PMID: 12520470.
Article
4. Chin KY, Tang MY. 1949; Congenital adenomatoid malformation of one lobe of a lung with general anasarca. Arch Pathol (Chic). 48:221–9. PMID: 18137795.
5. Stocker JT, Madewell JE, Drake RM. 1977; Congenital cystic adenomatoid malformation of the lung. Classification and morphologic spectrum. Hum Pathol. 8:155–71. DOI: 10.1016/S0046-8177(77)80078-6. PMID: 856714.
6. Davenport M, Warne SA, Cacciaguerra S, Patel S, Greenough A, Nicolaides K. 2004; Current outcome of antenally diagnosed cystic lung disease. J Pediatr Surg. 39:549–56. DOI: 10.1016/j.jpedsurg.2003.12.021. PMID: 15065026.
Article
7. Cacciari A, Ceccarelli PL, Pilu GL, Bianchini MA, Mordenti M, Gabrielli S, Milano V, Zanetti G, Pigna A, Gentili A. 1997; A series of 17 cases of congenital cystic adenomatoid malformation of the lung: management and outcome. Eur J Pediatr Surg. 7:84–9. DOI: 10.1055/s-2008-1071060. PMID: 9165253.
Article
8. Chikkannaiah P, Kangle R, Hawal M. 2013; Congenital cystic adenomatoid malformation of lung: report of two cases with review of literature. Lung India. 30:215–8. DOI: 10.4103/0970-2113.116272. PMID: 24049259. PMCID: PMC3775204.
Article
9. Patil AG, Karangadan S, Kishore V. 2015; Congenital cystic adenomatoid malformation of lung in fetus: report of two cases with brief review of literature. J Assoc Chest Physicians. 3:53–6. DOI: 10.4103/2320-8775.158854.
Article
10. Annam V, Korishetty SI, Yelikar BR, Hippargi SB, Shivalingappa DB. 2010; Bilateral congenital cystic adenomatoid malformation, stocker type III with associated findings and review of literature. Indian J Pathol Microbiol. 53:331–3. DOI: 10.4103/0377-4929.64324. PMID: 20551549.
Article
11. Boulot P, Pages A, Deschamps F, Hedon B, Laffargue F, Viala JL. 1991; Early prenatal diagnosis of congenital cystic adenomatoid malformation of the lung (Stocker's type I); a case report. Eur J Obstet Gynecol Reprod Biol. 41:159–62. DOI: 10.1016/0028-2243(91)90094-2.
Article
12. Sadler TW. Sadler TW, editor. 2012. Respiratory system. Langman's Medical Embryology. 12th ed. Lippincott Williams & Wilkins;Philadelphia: p. 202–6. DOI: 10.5005/jp/books/10252_13.
13. Ostör AG, Fortune DW. 1978; Congenital cystic adenomatoid malformation of the lung. Am J Clin Pathol. 70:595–604. DOI: 10.1093/ajcp/70.4.595. PMID: 360825.
Article
14. Moerman P, Fryns JP, Vandenberghe K, Devlieger H, Lauweryns JM. 1992; Pathogenesis of congenital cystic adenomatoid malformation of the lung. Histopathology. 21:315–21. DOI: 10.1111/j.1365-2559.1992.tb00401.x. PMID: 1398534.
Article
15. Liechty KW, Crombleholme TM, Quinn TM, Cass DL, Flake AW, Adzick NS. 1999; Elevated platelet-derived growth factor-B in congenital cystic adenomatoid malformations requiring fetal resection. J Pediatr Surg. 34:805–9. discussion 809–10. DOI: 10.1016/S0022-3468(99)90377-9. PMID: 10359185.
Article
Full Text Links
  • ACB
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2024 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr